What Is Ehlers-Danlos Syndrome?

April 16, 2007

In response to a few questions, I’d like to help out my friends and family in understanding what Ehlers-Danlos Syndrome (EDS) can do to a person. This may also help friends understand why I have physical limitations and what those limits may be. It will also help me by reducing the time I spend responding to questions that often go over the same ground.1. EDS – What Is That?Ehlers-Danlos Syndrome (EDS) is a genetic disorder to results in weakened connective tissue. Connective tissue is the glue that holds the body together (think of it as the Force, Luke. It holds the universe together). It is in your ligaments, your skin, blood vessels, your stomach and your bowel, your uterus and your eyes. In short in anything that is not bone.

2. What Does EDS Do To The Body?
When you have EDS it can impact a large portion of the body:

– Your ligaments – and thereby your joints. Without stable ligaments, joints slip and slide in and out of places resulting in micro-trauma, subluxations (it pops back in by itself) or dislocations (you need help resetting). This can include your ankles, your knees, your hips, your wrists, you shoulders, your back, your neck and even your jaw
– Your eyes – retinal detachment and myopia
– Your jaw/teeth – TMJ and loss of teeth (after all, your teeth are rooted in place via ligaments). Periodontal disease is common
– Your heart and your arteries – death by aneurysm is common in one EDS variation where the average life expectancy is 50. In other forms of EDS, low blood pressure and fainting occurs because your blood vessels ‘stretch’
– Your stomach (some patients cannot tolerate aspirin or ibuprofen – the stomach wall lining is too fragile). Bowel ruptures can occur. Constipation and other motility (movement) disorders. Gastroparesis (paralysis of stomach = inability to process food)
– Your uterus (again possible ruptures) , pregnancy complications and urinary incontinence
– Often your ribs flex and dislocate or the lining next to your lungs is inflamed
– Your back
– Your skin – poor wound healing and scarring. Heavy stretch marks (something I had even as pre-teen with no history of weight lost/gains).
– your throat – inability to swallow and acid reflux
(I am sure there are more, but let’s keep moving)

Now luckily, not all of these symptoms happen to every patient – in fact there are 6 types of EDS. There is only one genetic test for one the 6 types (the most severe form called Vascular EDS or Type 4). The other types are matched by doctors as best they can via observation and detailed medical history.

3. What Have I Got?
In my case, when I was diagnosed a few years ago, I was told I had ‘benign hypermobility syndrome”. They called it benign because it was not life threatening. I had suffered for 7 years from dislocations and unexplained joint pain- first in one knee, then the other. It then spread to my hip, my ankles, my shoulder and then my wrists. After being in physical therapy constantly for 7 years, I was finally referred to a rheumatologist, diagnosed and then told there was nothing they could for me. (“You could have the thighs of Arnold Schwarzenegger and it still would not fix your knee problem” Okaaay?, I thought. Then I realized: eeeeew!)

At the Mayo Clinic, I was able to get a more definite diagnosis – because of some skin involvement, I was told I had “EDS Type 3 (hypermobility).” I was once again told that this was not life threatening, but I needed to have an echocardiogram every 5 years (for the possibility of aortic rupture) and I might experience severe osteoarthritis later. In the meantime, practice good ergonomics, do my physical therapy and take anti-inflammatories. I should expect to have a normal life span.

4. Ooops, They Didn’t Tell Me
What I didn’t expect was that I would start to experience some of those other symptoms (beyond the joint pain.)

In the past three years I have experienced:

-Joint pain and dislocations in every large and small joints in my body (except for my right wrist. Go right wrist. Go. Or rather don’t go. Just…stay in place).
– Loss of muscle tone as a result of not being able to walk or exercise without pain
-Gastritis from use of anti-inflammatories.
-Gastropareis (inability to process foods) – first as a result of taking one or two pain pills, now on regular basis
-inability to take any pain meds regularly due to said gastropareis/gastritis
-jaw/teeth problems

5. On The Daily Level
What this means on a practical basis is the following:

-If I sit too long (more than 20) min, my knees begin to slide out of position and start to hurt
-If I sit with straight legs (say in recliner), my lower back is strained
-If I stand or walk for more than 20 min, my muscles strain so hard to keep the joints in place that they become fatigued. Then I experience hip, knee, and ankle pain.
-My typing skills are poor because of wrist and finger problems
-I cannot sleep the night through – because when I roll onto my shoulders they shift out of place (and I wake in pain)
-I have switched to a softer diet to reduce the strain on my jaw. I often eat strained, liquid or soft food to help with digestion
-I have no consistent and effective means of pain control
-Driving is tough because my knees and shoulder and wrists hurt. Sitting as passenger in the car is also painful.
-I often get cranky and tired and confused from the pain – I misspeak and forget things as my body struggles to process the pain signals and the lack of sleep

6. But…But…But … I Saw You Doing X Just This Morning!
Well it boggles me too. I still look normal, I often feel OK and I can move and walk and lift with seemingly little problem. But as the days wear on, the pain increases because my joints and bones are shifting and slipping in ways too small to notice. Sometimes I wake up the next day in excruciating pain with no real link to what I’ve done the day before. This makes it very hard to avoid doing what might cause pain.

7. On Rubber Bands And Grandparents
One EDS patient explained it to me this way: ‘our bodies are like elastic waistbands or rubber bands. After being stretched over and over again they lose their strength and give way.’

If you look at the list above, you may also recognize symptoms that your grandparents or older relatives are experiencing as they enter their 70s and 80s (urinary incontinence, constipation, retinal detachment – those granny ads on TV make sense now). . ‘Sometimes having EDS is like being an 80 year old in a 43 year old woman’s body.’

I am considered one of the lucky ones – having the mild or ‘benign’ form. Something for which I am grateful every day. Really.

What I’ve just written is not the definitive or even medically accurate overview of EDS. I’ve oversimplified many medical issues to help clarify my points. More can be found here.

(c) 2006-2007

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20 comments

hi my name is ashley, im 18 yrs old and are experiencing some of those symptoms. i cant lift heavy things for too long, like even carrying groceries inside it hurts my hands terribly. i bruise extremely easy, my skin is very soft and is somewhat stretchy. i went to the dr recently an had blood test done to see y i bruise this easy an the dr. said im fine. which is not the case. i have a foot problem which is not rarely inherited on my side of the family and its not just a mild case. i know something is wrong and i think this is what it is.

Hi Ashley, if you’re looking for more information about EDS patients (or are looking for other patients with EDS), we always suggest the national foundations as an excellent starting point. In the US, the Ehlers-Danlos National Foundation and in the UK there is the Hypermoblity Organization. Links to both organizations can be found in the column to the right.

Dear Ashley-
At 18, I was just at the most mild, beginning symptoms, so you COULD have EDS and not be showing a lot of the symptoms yet. I would recommend going to http://www.EDNF.org, which is the national foundation for EDS. If they don’t have the answer for you there, go to facebook, and then search either
EHLERS-DANLOS SYNDROME, OR EHLERS-DANLOS NATIONAL FOUNDATION. That will hitch you up w/all kinds of patients, family members, etc. Someone will have the answer…you can also facebook me too, if you want. i have had the symptoms since age 6, and I am 49 now, so in the severe stages now, and unable to work, etc. So, have a lot of time!!

i have eds, my dr is ignorant of the condition and tells me there is nothing wrong, she refused my disability application for my 7 yr old who is very bad with disclocations and pain and general mobility. Finally though we have been referred for her to a fantastic ot who is in turn helping me! but i am so tired and in constant pain, i feel like death walking…but get this my dr says im a healthy woman get over it…..

Well, Kristinna, WELCOME TO THE EDS CLUB! We all have experienced this, or something like it, as we went through trying to get diagnosed, and trying to find a doc who really knew anything about it. I even found one who claimed to “know all about EDS…” yet, he was adamant that “EDS does NOT cause pain” !! Ha, ha!! What a joke!!! I left that guy behind real fast. Keep looking if you don’t have a doc who treats you properly yet. The EDS national foundation can be found at their website or facebook (www.EDNF.org OR
facebook.com/EHLERS-DANLOS NATIONAL FOUNDATION). They have a list of docs and may have someone in your area. If not, ask one of the many EDS patients you’ll find on facebook…one of us will have an answer, or try to help you find one. You can also look at facebook under EHLERS-DANLOS SYNDROME. Best of luck, and contact me, if you want. I’m on facebook and e-mail.

Thanks for this article. I am a family medicine doctor and finally got diagnosed last year. Sometimes it just really helps to find a cause of the symptoms. to know we are not crazy or “MAKING IT UP”. Also of note the hyper-mobility does not always lead to increased flexibility. This is what confused my diagnosis. I have muscle spasms around my joints which doesn’t allow for the classic signs(ie my thumbs dislocate but yet I can’t touch my thumb to my wrist).

That’s exactly what happened to me. My thumbs are extremely hyper mobile laterally and my geneticist told me he’d never seen thumbs like mine but since they didn’t touch my wrist he didn’t count it on the flexibility scale. Also I am an amateur bowler so my left hip subluxes a lot so I wasn’t able to bed over and touch the ground at my appt because I had bowled that morning and he didn’t count that either. He said that I had all the other sx of EDS III but my flexibility ruled it out and he dxed me with HMS. But I also have dyautomia (bradycardia and hypotension which causes a lot of vasovagal reactions), mod/severe gastrointestinal issues, dental crowding, lovely skin that doesn’t like to heal well, occipital neuralgia etc. the condition I can live with but the constant judging and doubt from others is not cool. Not just a year ago I used to be able to bowl 10 games in a row no stopping, now, I can bowl 1 game with out pain and I just push through the last two for league because I usually sublux my left hip in the first game. As I said though, the condition I can live with…it’s the cards I was dealt and its not anything I can’t handle. But the lack of support…that’s another story.

I also have eds hypermobility. I feel sorry for anyone who has live like we do. I have every thing you said and more. I can’t get disability, my dr think eds is a joke. I wish dr’s would educate themselves.

Hi Im Rachel Im 20 and I to have EDS type 3 it is so painful and my pain management doctor tells me well EDS doesnt hurt your just flexible, so its in your head. They think Im like a drug addict. I wanna say like hell it does you try being a 20 year old who has never led a normal life because Of constant pain i did not even have a chance to finish high school, lost all my friends and basically bed bound and if not bed bound at least home bound for months on end. Why would anyone choose to have no life. Anyways I do not only have EDS I also suffer from server Postural Orthostatic tachycardia syndrome and most of the time when I get up or walk or even change positions my heart rate gets up into the 150s or higher. I was actually diagnosed with POTS at 17 and just recently within the last year diagnosed with EDS. Im also being worked up for Chiari 1 malformation. They are almost positive I have it and that mean brain surgery. So anyways thanks for posting your story it helps for me anyways to know that Im not alone and that there are others out there who know what Im going through and know the pain it causes both physically and mentally.

Oh my…I am 44 and have suffered with knee dislocations since the age of 12. After many surgeries, and many failures, I was informed that EDS was my problem. Just to know that I am not CrAzY when I would complain of a new “joint ailment location” each day, is reassuring (I think)
I have woken from unrelated surgeries only to discover my patella was on the side of my knee. Dislocations are quite frequent and my chiropractor has played a big part in keeping joints where they should be.
What do you do for pain management? I use high doses of Ibuprofen and Tramacet, but find it difficult to sleep.
Now, I can safely say, my right ankle and a couple of fingers are the only joint NOT affected. My cartilage beds are flaking away, leaving dibre to get caught in my joints (knees predominately) causing the need for an annual double knee arthroscopic clean up. (Scheduled in 3 weeks time)
I just want to thank you for your simplistic description of this debilitating disease. It is so very difficult to describe day to day survival to someone unfamiliar with ailment…and I am tired.

I am 29 and was diagnosed a few years ago with EDS I have to have hip replacement due to the osteoarthrits. I have severe heart arrythmia’s they are trying to control. They may put in a pacemaker. I have scoliosis, degenerative disc disease, facet joint degeneration, bone spurs, osteomalacia, mitral valve prolapse, hypertrophic uterus, endometrial hyperplasia,syncope, hypotension, and i broke my pubic bone and didnt realize it cause i’m always in pain and just figured it was normal. I have tried getting disability only to be denied. I don’t think they truely understand what EDS can do to a person’s body. So I try to spread awareness and pray for a cure.

I am from a family of ehlers danlos sufferers.. I am one of 5 whom all have the condition and all my neices and nephews do too – 7 of them.

One of which (shes only 5)has been taken into hospital severely ill with gastroparesis of which there could be two underlying possibilities… diabetes (running in her dads side of the family) and the other is the EDS – the question I want to know is, what type of EDS would cause Gastroparesis as if it is the vascular one we need to know.

She also had a head injury at school 2 weeks ago which we think hid this issue as it started around the same time…all she did was bang heads with another child at school, but it caused her brain to bleed as a consequence – no swelling or anything the blood vessel just started bleeding… we really want to know what to make of all of this as we are so worried about her and the doctors are finding it hard to stop her vomiting.. she has been sick for the past 2 weeks and lost 5lbs already (and for a child of her height, weight and age – is not good in the slightest!)

When I was 10, I told my mother god gave me the joints of an 80 year old. I hurt all the time. My parents blamed me doing advanced gymnastics since I was a small child. I broke my elbow joint at age 12 falling off a trampling and hyperextending it till the ball joint shattered and snapped off. I joined the military at age 26. Injured my shoulder had had 4 unsuccessful surgeries. Tore the ligaments completely in my right ankle to where I had to learn to walk using my muscles to hold my joint in place.
My Dr finally diagnosed me with ehlers danlos hypermobilty syndrome. I made a perfect score on the test. Lol. I am pregnant with my 4th child and have no stretch marks. I figured the ED helped my skin stretch and bounce back. Days after giving birth I look like I was never pregnant.
But I agree people can be unfair. You look fine. One day you can say, I cant lift that grocery sack or my arm will come out of place, the next day your moving furniture. I don’t know why its like that. sitting for extended periods, like a movie or road trip is painful because my joints shift out of place. I have a high pain tolerance.
Something I wondered was related to ED though, my growth plate in my knees didn’t change till after I was 26. Which means I grew from 5 foot 1 at age 18, to 5 foot 5 at age 26. That’s not supposed to be possible.
I had little pain and less dislocation when I was a gymnast and an athlete. My muscles did hold me into place. But I had very large strong muscles. When I joined the military, I actually was LESS active then I was as an athlete, so my muscles wasted away and I was easily injured. So I would say work out. The way I worked out was swimming and using my own body weight as resistance. Hand stands to hand stand push ups, squatting against a wall. Laying on my back and lifting my feet 6 inches from the ground for 2 minutes or during commercials, etc. That’s how I became fit. I stretched every day. I must stretch before activity.
One wish I had was that there were physical therapists that specialize in ED patients. After surgery, when I would start physical therapy, I would get worse and have damage. They just don’t understand that muscle fatigue causes dislocation when your ligaments are healing. My last surgery on my shoulder, my orthopedic Dr made it mandatory that I did not do physical therapy. I didn’t need it I found out. I gained back range of motion without any physical therapy! My shoulder still comes out and shifts if I sleep on that side. But its much better. My Dr wanted to use cadivar (sp) ligaments and tendons but said my cells would take over it and they would become too lax also.
It’s such an interesting disorder but sucks to live with. 2 of my daughters have it. One is 15 and a talented dancer, the other is 2. Her geneticist said since she is also small stature on top of having ED, she has the perfect body type to become an Olympic gymnast. But then next she said, pity she can’t, it would damage her joints.
Meeting someone with ED is so fulfilling and validating. Sometimes you just feel crazy or think maybe your just a wimp. Then you hear their stories and get excited. Good luck to you all!

Have you ever been on Fibromyalgia medicine? For years I went to drs for pain and they never provided me with any pain management nor relief. Eventually I went to a different dr, a rheumatologist, and told him that I’m starting to wonder if it might be Fibromyalgia and EDS joint pain. Turns out that Lyrica works better than percocet, which barely took the edge off. He also said that most of his patients with EDS also have Fibromyalgia and that it seems common to have both. I’m 25 and I feel like my body is around 70-80ish.

This is a nitpick, but it’s going to bug me if I don’t bring it up. Bones are considered part of the connective tissues, and are in fact packed with collagen. They’re essentially calcified cartilage. Osteogenesis imperfecta, also known as “brittle bone disease” is a collagen disorder. Things that aren’t connective tissue are: nerve tissue, muscle tissue, or epithelial.

I’m looking into the possibility of having Ehler’s-Danlos. As I was typing this the doorbell rang. On my way back I stepped on something I left on the floor (22 year old male here 😉 and my ankle rolled, painlessly. I’ve been rolling my ankles since I was young and actually never sprained them.

I also have some odd scarring on my knees, stretch marks unexplained by weight gain or growth, etc.

I give in,yet why,it is real,not all in my head,my GP thinks how flexible I am,informs me he cant do what I can do,well I cant do what he can do. People think I am a freak,EDS what the hell is that,now my daughter having bruising,bleeding disorder,she is very bendy,The eds people say there is no connection . Help I want to crawl into a nice warm hole and nothing. yet I have given my legacy to my children,and so it goes . I have spasms,its my back and neck,I have no respect for anyone who says all I need to do is exercise to strenghten muscles,oh arreflexic,not clinical significance,do the blood tests,fine,.why use a wheelchair,cos if I didnt would not get out at all. Fed up of being lectured to,I know my body,know what I feel,wish I could transfer it to anyone who views me with sceptism and disbelief. sorry shouldnt go on you all know only to well,try telling anyone,anti depressants hah,gave them to my mother when she said she was unwell,then she died due to undiagnosed heart problem ,

I have EDS. I find that I have so many symptoms that my doctor couldn’t help but diagnose it. It doesn’t affect my joints too much, but I am unusually stretchy. Thanks for blogging about this. The way it affects each person can be quite unique, so it is nice to find a forum.

Thanks for writing this. I always find it difficult to share with people whats going on with my body, so I like it when other people write it out. And I think people take me more seriously when I’m not the only one experiencing these things. I stumbled across this while looking for eds and dental forums. Though I take very good care of my teeth, they are breaking into pieces and boy does it hurt. I am 24 and I cannot walk most of the time, I cannot pick up my nephews, and I cannot live alone anymore because so much has broken down… the least my body can do is leave me my teeth. On the plus side, I can get some fancy falsies and maybe my smile will be better than it was with real teeth.

I don’t know if I have EDS or not. My sister said it runs in our family thru our mother’s side her kids are the only ones I know of in our family who have elastic skin & hypermobility. I never took it seriously since I didn’t think I had it. But suddenly, in a matter of a few short days, I suddenly developed massive stretch marks in my groin & hip area. The Mayo Clinic’s website had listed EDS as a possible cause so I eventually ended up on this site. I was in a car accident 7 months ago that caused several bulged discs throughout my back & neck. I had steroid injections two months ago and it did more damage than it helped. I now have the most insane muscle tightness and I don’t last long sitting or standing before my body shuts me down. One day I can bend a bit and do a lot & the next I can’t. In the morning I feel totally capable but by noon I am pushing to get through the day. I have had to live on ibuprofen & now have acid reflux. I try not to take any muscle relaxers unless I absolutely need them & even then I half or quarter them. Physical therapy helps but my first PT specialized in vestibular & would scold me if I was in pain and was eager to get rid of me. I started at a more understanding physical therapy office only to find out they have a policy that limits visits on attorney liens. I have always had popping joints (sternum, hips, shoulders, wrists, ankles, knees, etc.) but not dislocated joints but I usually stretch a bit beyond the norm. What I feel is a good stretch seems to feel good to some muscles but makes others worse in the process. And I get pains that I haven’t a clue where they came from but can be very debilitating. It is very depressing. Even if I don’t have EDS, reading about the similar experiences & pain helps me feel I am not alone.